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Antenatal Bartter syndrome

MedGen UID:
893350
Concept ID:
CN239220
Disease or Syndrome
Related genes: MAGED2, SLC12A1, KCNJ1

Definition

Bartter syndrome is a group of very similar kidney disorders that cause an imbalance of potassium, sodium, chloride, and related molecules in the body.

Two major forms of Bartter syndrome are distinguished by their age of onset and severity. One form begins before birth (antenatal) and is often life-threatening. The other form, often called the classical form, begins in early childhood and tends to be less severe. Once the genetic causes of Bartter syndrome were identified, researchers also split the disorder into different types based on the genes involved. Types I, II, and IV have the features of antenatal Bartter syndrome. Because type IV is also associated with hearing loss, it is sometimes called antenatal Bartter syndrome with sensorineural deafness. Type III usually has the features of classical Bartter syndrome.

In some cases, Bartter syndrome becomes apparent before birth. The disorder can cause polyhydramnios, which is an increased volume of fluid surrounding the fetus (amniotic fluid). Polyhydramnios increases the risk of premature birth.

Beginning in infancy, affected individuals often fail to grow and gain weight at the expected rate (failure to thrive). They lose excess amounts of salt (sodium chloride) in their urine, which leads to dehydration, constipation, and increased urine production (polyuria). In addition, large amounts of calcium are lost through the urine (hypercalciuria), which can cause weakening of the bones (osteopenia). Some of the calcium is deposited in the kidneys as they are concentrating urine, leading to hardening of the kidney tissue (nephrocalcinosis). Bartter syndrome is also characterized by low levels of potassium in the blood (hypokalemia), which can result in muscle weakness, cramping, and fatigue. Rarely, affected children develop hearing loss caused by abnormalities in the inner ear (sensorineural deafness). [from MedlinePlus Genetics]

Professional guidelines

PubMed

Walsh CJ, Micke K, Elfman H, Bock M, Harper T, Zaretsky M, Galan HL, Behrendt N, Putra M
Prenat Diagn 2024 Feb;44(2):172-179. Epub 2023 Dec 30 doi: 10.1002/pd.6508. PMID: 38159268

Recent clinical studies

Etiology

Hernández NEG, Pérez LIE, Aguilera D, Camargo-Muñiz MD, Espinosa CFC, Jaramillo MCR, Salvador C, González ZL, Hureaux M, Vargas-Poussou R
Arch Med Res 2023 Sep;54(6):102859. Epub 2023 Jul 27 doi: 10.1016/j.arcmed.2023.102859. PMID: 37516009
Spector-Cohen I, Koren A, Sakran W, Tenenbaum-Rakover Y, Halevy R
J Pediatr Endocrinol Metab 2019 Mar 26;32(3):225-231. doi: 10.1515/jpem-2018-0188. PMID: 30844761
Legrand A, Treard C, Roncelin I, Dreux S, Bertholet-Thomas A, Broux F, Bruno D, Decramer S, Deschenes G, Djeddi D, Guigonis V, Jay N, Khalifeh T, Llanas B, Morin D, Morin G, Nobili F, Pietrement C, Ryckewaert A, Salomon R, Vrillon I, Blanchard A, Vargas-Poussou R
Clin J Am Soc Nephrol 2018 Feb 7;13(2):242-250. Epub 2017 Nov 16 doi: 10.2215/CJN.05670517. PMID: 29146702Free PMC Article
Shalev H, Ohali M, Kachko L, Landau D
Pediatrics 2003 Sep;112(3 Pt 1):628-33. doi: 10.1542/peds.112.3.628. PMID: 12949294
Birkenhäger R, Otto E, Schürmann MJ, Vollmer M, Ruf EM, Maier-Lutz I, Beekmann F, Fekete A, Omran H, Feldmann D, Milford DV, Jeck N, Konrad M, Landau D, Knoers NV, Antignac C, Sudbrak R, Kispert A, Hildebrandt F
Nat Genet 2001 Nov;29(3):310-4. doi: 10.1038/ng752. PMID: 11687798

Diagnosis

Takemori S, Tanigaki S, Nozu K, Yoshihashi H, Uchiumi Y, Sakaguchi K, Tsushima K, Kitamura A, Kobayashi C, Matsuhima M, Tajima A, Nagano C, Kobayashi Y
Eur J Med Genet 2021 Oct;64(10):104308. Epub 2021 Aug 13 doi: 10.1016/j.ejmg.2021.104308. PMID: 34400373
Akther M, Moni SC, Jahan I, Shabuj KH, Dey SK, Mannan MA, Shahidullah M
Mymensingh Med J 2020 Apr;29(2):469-472. PMID: 32506108
Kömhoff M, Laghmani K
Curr Opin Nephrol Hypertens 2017 Sep;26(5):419-425. doi: 10.1097/MNH.0000000000000346. PMID: 28598867
Narayan R, Peres M, Kesby G
Clin Exp Obstet Gynecol 2016;43(3):453-4. PMID: 27328514
Afzal M, Khan EA, Khan WA, Anwar V, Yaqoob A, Rafiq S, Azam S
J Coll Physicians Surg Pak 2014 May;24 Suppl 2:S121-3. PMID: 24906263

Therapy

Spector-Cohen I, Koren A, Sakran W, Tenenbaum-Rakover Y, Halevy R
J Pediatr Endocrinol Metab 2019 Mar 26;32(3):225-231. doi: 10.1515/jpem-2018-0188. PMID: 30844761
Narayan R, Peres M, Kesby G
Clin Exp Obstet Gynecol 2016;43(3):453-4. PMID: 27328514
Afzal M, Khan EA, Khan WA, Anwar V, Yaqoob A, Rafiq S, Azam S
J Coll Physicians Surg Pak 2014 May;24 Suppl 2:S121-3. PMID: 24906263
Reinalter SC, Jeck N, Peters M, Seyberth HW
Acta Physiol Scand 2004 Aug;181(4):513-21. doi: 10.1111/j.1365-201X.2004.01325.x. PMID: 15283765
Shalev H, Ohali M, Kachko L, Landau D
Pediatrics 2003 Sep;112(3 Pt 1):628-33. doi: 10.1542/peds.112.3.628. PMID: 12949294

Prognosis

Gross I, Siedner-Weintraub Y, Simckes A, Gillis D
J Pediatr Endocrinol Metab 2015 Jul;28(7-8):943-6. doi: 10.1515/jpem-2014-0188. PMID: 25741940
Afzal M, Khan EA, Khan WA, Anwar V, Yaqoob A, Rafiq S, Azam S
J Coll Physicians Surg Pak 2014 May;24 Suppl 2:S121-3. PMID: 24906263
Sharma PK, Saikia B, Sharma R, Ankur K, Khilnani P, Aggarwal VK, Cheong H
Indian J Pediatr 2014 Oct;81(10):1095-8. Epub 2014 Apr 4 doi: 10.1007/s12098-014-1379-6. PMID: 24696311
Puricelli E, Bettinelli A, Borsa N, Sironi F, Mattiello C, Tammaro F, Tedeschi S, Bianchetti MG; Italian Collaborative Group for Bartter Syndrome
Nephrol Dial Transplant 2010 Sep;25(9):2976-81. Epub 2010 Mar 10 doi: 10.1093/ndt/gfq119. PMID: 20219833
Reinalter SC, Jeck N, Peters M, Seyberth HW
Acta Physiol Scand 2004 Aug;181(4):513-21. doi: 10.1111/j.1365-201X.2004.01325.x. PMID: 15283765

Clinical prediction guides

Walsh CJ, Micke K, Elfman H, Bock M, Harper T, Zaretsky M, Galan HL, Behrendt N, Putra M
Prenat Diagn 2024 Feb;44(2):172-179. Epub 2023 Dec 30 doi: 10.1002/pd.6508. PMID: 38159268
Spector-Cohen I, Koren A, Sakran W, Tenenbaum-Rakover Y, Halevy R
J Pediatr Endocrinol Metab 2019 Mar 26;32(3):225-231. doi: 10.1515/jpem-2018-0188. PMID: 30844761
Breinbjerg A, Siggaard Rittig C, Gregersen N, Rittig S, Hvarregaard Christensen J
Acta Paediatr 2017 Jan;106(1):161-167. doi: 10.1111/apa.13635. PMID: 27748541
Puricelli E, Bettinelli A, Borsa N, Sironi F, Mattiello C, Tammaro F, Tedeschi S, Bianchetti MG; Italian Collaborative Group for Bartter Syndrome
Nephrol Dial Transplant 2010 Sep;25(9):2976-81. Epub 2010 Mar 10 doi: 10.1093/ndt/gfq119. PMID: 20219833
Reinalter SC, Jeck N, Brochhausen C, Watzer B, Nüsing RM, Seyberth HW, Kömhoff M
Kidney Int 2002 Jul;62(1):253-60. doi: 10.1046/j.1523-1755.2002.00435.x. PMID: 12081585

Recent systematic reviews

von Vigier RO, Ortisi MT, La Manna A, Bianchetti MG, Bettinelli A
Pediatr Nephrol 2010 May;25(5):861-6. Epub 2009 Dec 22 doi: 10.1007/s00467-009-1388-2. PMID: 20033223

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